Lin Yan-Ting, Lin Chiung-Hung, Wang Shao-Chung, Huang Yun-Ju, Wu Ren-Chin, Lee Chung-Shu, Chang Chih-Hao
Department of Internal Medicine, Chang Gung Memorial Hospital, School of Medicine, Chang Gung University, Taipei, Taiwan.
Department of Thoracic Medicine, Chang Gung Memorial Hospital, School of Medicine, Chang Gung University, Taipei, Taiwan.
J Med Ultrasound. 2022 Oct 7;31(4):314-317. doi: 10.4103/jmu.jmu_56_22. eCollection 2023 Oct-Dec.
We report a patient with systemic sclerosis who was diagnosed with advanced-stage mucinous adenocarcinoma of the lungs. The clinical presentation, imaging findings, pathological results, and molecular diagnoses are presented. A 64-year-old woman with systemic sclerosis was administered prednisolone and hydroxychloroquine sulfate to control her disease. High-resolution computed tomography (HRCT) revealed an interstitial pattern in both lungs during annual imaging. Connective tissue disease-associated interstitial lung disease (CTD-ILD) was diagnosed using blood tests, pulmonary function tests, and imaging findings. One year later, the patient underwent follow-up chest HRCT, which showed progressive lung disease. The patient underwent endobronchial ultrasound (EBUS)-guided transbronchial lung cryobiopsy and computed tomography-guided biopsy for a pathological diagnosis. The pathology reports of bilateral lungs disclosed mucinous adenocarcinoma. After tumor staging and mutation testing, the patient received chemotherapy with pemetrexed and cisplatin. The bilateral lung lesions subsided after four cycles of first-line chemotherapy. Patients with CTD and lung involvement may be diagnosed with CTD-ILD. Although histopathological results are not mandatory for ILD diagnosis, EBUS-guided transbronchial lung biopsy or lung cryobiopsy should be considered when ILD has atypical or unexplained features.
我们报告了一名系统性硬化症患者,其被诊断为晚期肺黏液腺癌。本文呈现了该患者的临床表现、影像学检查结果、病理结果及分子诊断情况。一名64岁患有系统性硬化症的女性接受泼尼松龙和硫酸羟氯喹治疗以控制病情。年度影像学检查时,高分辨率计算机断层扫描(HRCT)显示双肺呈间质性改变。通过血液检查、肺功能检查及影像学检查结果诊断为结缔组织病相关性间质性肺病(CTD-ILD)。一年后,患者接受胸部HRCT随访,结果显示肺部疾病进展。患者接受了支气管内超声(EBUS)引导下经支气管肺冷冻活检及计算机断层扫描引导下活检以明确病理诊断。双侧肺脏的病理报告显示为黏液腺癌。在进行肿瘤分期及突变检测后,患者接受培美曲塞和顺铂化疗。一线化疗四个周期后,双侧肺部病变消退。患有CTD且有肺部受累的患者可能被诊断为CTD-ILD。虽然ILD诊断并非必须有组织病理学结果,但当ILD具有非典型或无法解释的特征时,应考虑进行EBUS引导下经支气管肺活检或肺冷冻活检。